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BBS7 rabbit pAb

商品貨號(hào)： PLA020545

適應(yīng) 性： 人,小鼠

￥600元

規(guī)格：

40μL 100μL 200μL

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MSDS

說明書

商品描述

基因名稱： BBS7 BBS2L1
蛋白名稱： BBS7
Human_gene_id： 55212
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=55212
Human_swiss_prot_no： Q8IWZ6
Human_swiss_link： https://www.uniprot.org/uniprotkb/Q8IWZ6/entry
Mouse_gene_id： 71492
Mouse_gene_link： https://www.uniprot.org/uniprot/71492
Mouse_swiss_prot_no： Q8K2G4
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q8K2G4
特異性： This antibody detects endogenous levels of BBS7 at Human/Mouse
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來(lái)源： Polyclonal, Rabbit,IgG
稀釋： WB 1：500-2000
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 79kD
功能： disease:Defects in BBS7 are a cause of Bardet-Biedl syndrome type 7 (BBS7) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect. Inheritance is autosomal recessive, but three mutated alleles (two at one locus, and a third at a second locus) may be required for disease manifestation in some cases (triallelic inheritance).,function:The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane.,online information:Retina International's Scientific Newsletter,subcellular location:Localizes to nonmembranous centriolar satellites in the cytoplasm.,subunit:Part of BBSome complex, that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10. The BBSome complex binds to PCM1 and tubulin.,tissue specificity:Isoform 2 is ubiquitously expressed. Isoform 1 is expressed in retina, lung, liver, testis, ovary, prostate, small intestine, liver, brain, heart and pancreas.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Cell projection, cilium membrane . Cytoplasm . Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite . Cytoplasm, cytoskeleton, cilium basal body .
組織表達(dá)： Isoform 2 is ubiquitously expressed. Isoform 1 is expressed in retina, lung, liver, testis, ovary, prostate, small intestine, liver, brain, heart and pancreas.
科研貨號(hào)： PLA020545

BBS7 rabbit pAb

Catalog No PLA020545

Product information

基因名稱： BBS7 BBS2L1
蛋白名稱： BBS7
Human_gene_id： 55212
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=55212
Human_swiss_prot_no： Q8IWZ6
Human_swiss_link： https://www.uniprot.org/uniprotkb/Q8IWZ6/entry
Mouse_gene_id： 71492
Mouse_gene_link： https://www.uniprot.org/uniprot/71492
Mouse_swiss_prot_no： Q8K2G4
Mouse_swiss_link： https://www.uniprot.org/uniprotkb/Q8K2G4
特異性： This antibody detects endogenous levels of BBS7 at Human/Mouse
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來(lái)源： Polyclonal, Rabbit,IgG
稀釋： WB 1：500-2000
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲(chǔ)存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
分子量： 79kD
功能： disease:Defects in BBS7 are a cause of Bardet-Biedl syndrome type 7 (BBS7) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect. Inheritance is autosomal recessive, but three mutated alleles (two at one locus, and a third at a second locus) may be required for disease manifestation in some cases (triallelic inheritance).,function:The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane.,online information:Retina International's Scientific Newsletter,subcellular location:Localizes to nonmembranous centriolar satellites in the cytoplasm.,subunit:Part of BBSome complex, that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10. The BBSome complex binds to PCM1 and tubulin.,tissue specificity:Isoform 2 is ubiquitously expressed. Isoform 1 is expressed in retina, lung, liver, testis, ovary, prostate, small intestine, liver, brain, heart and pancreas.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Cell projection, cilium membrane . Cytoplasm . Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite . Cytoplasm, cytoskeleton, cilium basal body .
組織表達(dá)： Isoform 2 is ubiquitously expressed. Isoform 1 is expressed in retina, lung, liver, testis, ovary, prostate, small intestine, liver, brain, heart and pancreas.
科研貨號(hào)： PLA020545

Hunan UPT Biotechnology Co.,Ltd

Website：m.tjdqdt.com Servive hotline :4006916686

E-mail：service@uptbio.com

Address：

Room 402, Building 13, Xinggong International Industrial Park, 100 Guyuan Road, Yuelu District, Changsha City, Hunan Province, China.

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